Clinical characteristics, imaging findings, management, and outcomes of patients with scimitar syndrome at a tertiary referral healthcare center in Colombia.

Scimitar Syndrome is part of a complex spectrum of congenital cardiovascular anomalies related to anomalous pulmonary venous return. Depending on the extent of involvement, treatment can be either expectant or surgical. Prognosis and survival have been controversial, with some results supporting early surgical management. This research aims to disclose the outcomes and describe the management, clinical and imaging characteristics of patients diagnosed with Scimitar Syndrome treated in a tertiary referral healthcare center. Longitudinal descriptive observational study. The study included all patients diagnosed with scimitar syndrome in our institution between January/2011 and December/2022. A description of the sociodemographic and clinical characteristics, diagnostic tools used, treatment features, and patient outcomes is provided. Eleven patients were included, with a mean age at diagnosis of five years (CI 0-17), six of which were female (54.55%). Nine (81.82%) patients had evidence of a scimitar vein on the chest radiograph, six (54.55%) cardiac dextroposition, six (54.55%) pulmonary hypoplasia, five (45.45%) right pulmonary artery hypoplasia, and three (27.27%) had aortopulmonary collaterals. Four (36.36%) patients had horseshoe lungs, and four (36.36%) had bronchopulmonary sequestration. In the associations, two (18.18%) patients were found to have an atrial septal defect, three (27.27%) ventricular septal defect, and one (9%) had Tetralogy of Fallot. Pulmonary hypertension was demonstrated in two (18.18%) patients. Seven (63.64%) required surgical management to correct the scimitar vein, and two patients died due to unrelated complications. Scimitar syndrome presents diagnostic and treatment challenges, necessitating a multidisciplinary approach for timely care. Chest radiography and CT scans are primary diagnostic tools, with surgical intervention often warranted alongside other heart defects or significant hemodynamic repercussions. Medical management is effective for mild to moderate cases. Long-term patient outcomes remain uncertain due to study limitations, but improved life expectancy is anticipated with ongoing care.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Standardizing Radiation Dose Reporting in thePediatric Cardiac Catheterization Laboratory—AMulticenter Study by the CCISC (CongenitalCardiovascular Interventional Study Consortium)

We examine normalized air Kerma area product (PKA) by body weight(PKA/BW) as a reference value of radiation dose and benchmark PKA/BW in pediatriclaboratories using a multicenter registry database. Background: Reduction of radiationdose is an important quality improvement task in pediatric cardiac catheterizationlaboratories. Physicians need to agree on a standard method of reportingradiation dose that would allow comparisons to be made between operators andinstitutions. Methods: This was a multicenter observational study of radiation dosein pediatric laboratories. Patient demographic, procedural and radiation data includingfluoroscopic time and PKA (mGy m2) were analyzed. PKA/BW was obtained byindexing PKA to body weight. Results: A total of 8,267 pediatric catheterization procedures(age <18 years) were included from 16 institutions. The procedures consistedof diagnostic (n52,827), transplant right ventricular (RV) biopsy (n51,172),and interventional catheterizations (n54268). PKA correlated with body weight betterthan with age and best correlated with weight–fluoroscopic time product. PKA/BWshowed consistent values across pediatric ages. Interventional catheterizations hadthe highest PKA/BW (50th, 75th, and 90th percentiles: 72, 151, and 281 lGy m2/kg),followed by diagnostic (59, 105, and 175 lGy m2/kg) and transplant RV biopsy (27,79, and 114 lGy m2/kg). Conclusion: PKA/BW appeared to be the most reliablestandard to report radiation dose across all procedure types and patient age. We recommend PKA/BW to be used as the standard unit in documenting radiationusage in pediatric laboratories and can be used to evaluate strategies to lower radiationdosage in pediatric patients undergoing cardiac catheterizations.